Health-Related Quality of Life in Patients with MPS II.

Mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome, is a chronic and progressive X-linked lysosomal disease that mainly affects males. The National MPS Society (2013) reports that MPS II affects 1 in 100,000 to 1 in 150,000 males worldwide. Two distinct forms of the disease are based on age of onset and clinical course: attenuated and severe. MPS II affects many organ systems including the nervous, cardiovascular, gastrointestinal and respiratory systems. Clinical manifestations can include progressive hearing loss, mental impairment, and enlarged liver and spleen. This study focuses on the health-related quality of life of individuals (HRQOL) with MPS II as measured by the parent and self-report versions of the Pediatric Quality of Life Inventory (PedsQL™). Both parents of patients with MPS II as well as patients themselves reported lower scores on all domains of the PedsQL™ (physical, emotional, social and school functioning) indicating that children with MPS II have an overall lower HRQOL when compared to a healthy sample. When compared with patients with other chronic illnesses (cancer, MSUD, galactosemia,), the MPS II sample had significantly lower scores on a number of PedsQL™ scales, suggesting an overall lower HRQOL. No significant relationships were found using scores from parent or self report PedsQL™ measures and length of time on ERT.

MPS II: adaptive behavior of patients and impact on the family system.

Mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome, is a chronic and progressive X-linked lysosomal disease that mainly affects males. It occurs in 1 in every 65,000 to 1 in 132,000 births. There are two distinct forms of the disease based on age of onset and clinical course: mild and severe. MPS II affects many organ systems including the nervous, cardiovascular, gastrointestinal and respiratory systems. Complications can include vision problems, progressive hearing loss, thickened and elastic skin, mental impairment, and enlarged liver and spleen. We herein focus on the adaptive behavior of individuals with MPS II, and the impact of MPS II on the family system. Outcomes from the Vineland-II Adaptive Behavior Scales showed that the MPS II patient sample experienced significantly lower functioning in communication, daily living skills, socialization, and motor skills compared to normative data. Patients with severe MPS II were found to have significantly lower adaptive functioning in all domains, as compared to those with mild MPS II. Length of time on ERT had no significant relationship to adaptive functioning. Results from the Peds QL Family Impact Module indicated that families of patients with MPS II experienced a lower overall health-related quality of life and overall lower family functioning (including lower emotional and cognitive functioning) than those with chronic illnesses residing in an inpatient setting.

An exploration of patient experiences of transitioning to a remote day programme following inpatient treatment in an adult eating disorder service.

WHAT IS KNOWN ON THE SUBJECT?: Traditionally, treatment for eating disorders (EDs) has been provided on an inpatient or outpatient basis, but more recently other levels of treatment provision to include, day care (DC) and community outreach programmes, have been developed. There is limited research exploring the experience of patients who have transitioned from inpatient ED treatment to a remote DC treatment. This lack of knowledge can impact mental health nurses' understanding of what that experience is like for patients and hence may influence the efficacy of collaboration and inclusion between patients and nurses. WHAT THIS PAPER ADDS TO EXISTING KNOWLEDGE?: This research addresses this dearth of knowledge and adds to our understanding of patients' experiences of attending a remote DC programme following a period of inpatient treatment for an ED. This is an important study for nurses and other mental health professionals working with people in ED treatment, as it highlights the specific challenges and anxieties of transitioning from inpatient to a remote DC programme and the individualized supports advisable during this process. WHAT ARE THE IMPLICATIONS FOR PRACTICE?: This research provides a basis on which nurses can understand and address the challenges experienced by patients after transitioning to a less intensive supportive ED programme. The understanding of these experiences will enhance the therapeutic alliance between the nurse and patient, which will in turn support the patient in increasing agency as they progress through their recovery. This research provides a foundation on which to develop specific supports that need to be in place to help patients manage the anxieties they experience as they transition to a less intense and remote treatment. Findings from these lived experiences can be used to support the development of similar DC programmes for EDs in other settings. ABSTRACT: INTRODUCTION: Day care (DC) treatment for people with an eating disorder (ED) provides for an easier transition from hospital to home, along with a treatment milieu where patients can maintain occupational and social functioning and allow for the transfer of newly acquired skills to everyday life. AIM: To explore patients' experiences of attending a remote day programme following intense inpatient treatment in an adult ED service. METHOD: A qualitative descriptive methodology informed the study. In-depth semi-structured interviews were conducted with 10 consenting patients. A thematic analysis framework was used to guide the process of data analysis. RESULTS: Three themes reflected participants' experiences: 'Moving On: Preparing for Change', 'Navigating a New Support System' and 'Increasing Agency'. DISCUSSION: An underlying issue for participants was an ongoing but changing experience of anxiety. While anticipatory anxiety is evident in preparing for discharge, this is replaced with a real-time anxiety as they endeavour to negotiate an effective support network. IMPLICATIONS FOR PRACTICE: The findings from this study provide a basis on which mental health nurses can develop timely and effective treatment and support systems with patients who are transitioning from a high support inpatient ED programme to a less intensive ED remote DC programme.